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Rheumatic Heart Disease

Diseases Rheumatic Heart

Rheumatic heart disease refers to a group of heart disorders that develop as a complication of rheumatic fever. It is one of the most common heart disease in children, particularly in developing countries.


Rheumatic fever is the antecedent of rheumatic heart disease. As per the Jones criteria, rheumatic heart disease will have indication of group A streptococcal infection, with one or two major diagnostic criteria, and two minor ones [7]. The major diagnostic manifestations are

Minor manifestations of the disease are fever, arthralgia, long PR interval, and elevated acute phase reactant, elevated levels of leucocytes and presence of C-reactive protein.
In some patients, difficulty in breathing, exercise intolerance, and a rapid heart rate may indicate rheumatic heart disease.

  • BACKGROUND: Rheumatic fever and rheumatic heart disease (RHD) are important health problems in developing countries.[ncbi.nlm.nih.gov]
  • Rheumatic fever is caused by the infection of the bacteria streptococci. Rheumatic fever affects heart, joints and the central nervous system. When left untreated, it may lead to valve damage, and thus, heart failure and death.[symptoma.com]
  • Fever, anaemia, splenomegaly and positive blood cultures for Gram-negative bacteria were found on admission. The fever resolved with antibiotic therapy on the third hospital day but he then developed hemiplegia and multifocal seizures.[ncbi.nlm.nih.gov]
  • Furthermore the natural history of acute rheumatic fever in the Indigenous population is progressive requiring strict adherence to secondary penicillin prophylaxis.[ncbi.nlm.nih.gov]
  • Our patient is an 18-year-old Caucasian woman from the UK who developed severe mitral stenosis on a history of childhood acute rheumatic fever (ARF) and rheumatic heart disease (RHD).[ncbi.nlm.nih.gov]
Congestive Heart Failure
  • The seizures progressed to uncontrollable status epilepticus accompanied by congestive heart failure and the patient died 20 d after admission.[ncbi.nlm.nih.gov]
  • However, the incidence of congestive heart failure due to RHD in adults remains high ( 25/100 000/year) in Gauteng Province, and is associated with a high case fatality rate of up to 35% in 6 months.[ncbi.nlm.nih.gov]
  • Over a six-year period three patients with rheumatic valvular disease presented with congestive heart failure due to abnormalities in myocardial diastolic function.[ncbi.nlm.nih.gov]
  • Some patients may develop congestive heart failure with dyspnea, ascites, hepatomegaly and chest pain as indications. Chorea – this is characterized by choreiform movements which increase during stress.[symptoma.com]
  • Rheumatic heart disease chronically manifests as congestive heart failure from valvular involvement. Most commonly the mitral valve is affected, resulting in mitral stenosis or mitral regurgitation.[healio.com]
  • Symptoms of heart valve problems, which can result from rheumatic heart disease, include: Chest pain Excessive fatigue Heart palpitations Shortness of breath Swollen ankles, wrists or stomach Thumping /pounding sensation in the chest Rheumatic Heart Disease[baptisthealth.com]
  • Symptoms Symptoms of heart valve problems, which are often the result of rheumatic heart disease, can include: chest pain, excessive fatigue, heart palpitations (when the heart flutters or misses beats), a thumping sensation in the chest, shortness of[heartandstroke.ca]
  • […] your child's neuromuscular movements (this is usually noted by a change in your child's handwriting and may also include jerky movements) Rash (a pink rash with odd edges that is usually seen on the trunk of the body or arms and legs) Fever Weight loss Fatigue[chw.org]
  • To prevent fatigue. - Suggest parent be present during procedures. To comfort child Activity Intolerance related to joint pain.[slideshare.net]
  • Carditis can cause a rapid heart rate, fatigue, shortness of breath and exercise intolerance. This is the most serious of the symptoms and may have long-term effects on health.[chop.edu]
  • Critical Essential Core Tested Community Questions (3) (M2.CV.36) A 15-year-old girl presents with four days of malaise, painful joints, nodular swelling over her elbows, low-grade fever, and a rash on her chest and left shoulder.[medbullets.com]
  • Symptoms Symptoms of ARF include: fever malaise (feeling of being unwell) painful and/or swollen joints (one or many joints) – usually affecting the elbows, wrists, hips, knees and ankles chest pain, difficulty breathing, or a rapid heartbeat and heart[sahealth.sa.gov.au]
  • The most common indications for echocardiography were heart failure (47.6%), and dyspnea (42.9%). The mitral valve was the most commonly affected valve in 80.9% of cases.[ncbi.nlm.nih.gov]
  • If you have mitral stenosis, you may develop breathing difficulty (dyspnea); swelling of the ankle and feet (edema); and irregular heartbeats (arrhythmia). Your doctor will treat you with medications, if you have mild valve abnormality.[quest4health.com]
  • Some patients may develop congestive heart failure with dyspnea, ascites, hepatomegaly and chest pain as indications. Chorea – this is characterized by choreiform movements which increase during stress.[symptoma.com]
  • She coughed throughout the day. Her heart – weakened and scarred from an infection – could not effectively pump blood to the rest of her body. Louise was slowly suffocating.[pih.org]
  • Streptococci are spread when an infected person talks, coughs or sneezes small droplets containing infectious agents into the air. The droplets in the air may be breathed in by those nearby.[sahealth.sa.gov.au]
  • Physical Examination Physical examination findings include inflamed joints, subcutaneous nodules, a pericardial friction rub, findings of congestive heart failure (edema, pulmonary rales, elevated jugular venous pressure), and the rash of erythema marginatum[healio.com]
  • Fever, rash, headache, weight loss, nose bleeding, fatigue, excessive sweating, stomach pain, chest pain, new heart murmurs, shortness of breath, and vomiting are some of the general symptoms.[wkcardiology.com]
Abdominal Pain
  • These include: Joint pain and swelling Rash Small, hard, round bumps under the skin (nodules) Irregular or jerky movements Belly (abdominal) pain Bloody nose Fever The symptoms of rheumatic heart disease can be like other health conditions.[stlouischildrens.org]
Heart Disease
  • Global longitudinal strain was lower than the 5th percentile in 44% of the subjects with any rheumatic heart disease (p 0.002 versus controls) and 57% of the subjects with definite rheumatic heart disease (p 0.03).[ncbi.nlm.nih.gov]
  • Rheumatic heart disease refers to a group of heart disorders that develop as a complication of rheumatic fever. It is one of the most common heart disease in children, particularly in developing countries.[symptoma.com]
  • Results: A total of 324,676 patients were seen at the Paediatric unit of LASUTH from 2007 to 2016, out of which 36 had Rheumatic heart disease.[ncbi.nlm.nih.gov]
  • Carditis is a well-recognized finding in rheumatic heart disease and is one of the major criteria in the diagnosis of rheumatic fever.[ncbi.nlm.nih.gov]
  • Clinical officers had a high sensitivity in detecting rheumatic heart disease.[ncbi.nlm.nih.gov]
Chest Pain
  • A 44-year-old lady with rheumatic valvular disease and atrial fibrillation defaulted anticoagulant medication, and subsequently presented with acute chest pain, acute left ventricular failure, focal neurological deficit and gangrenous lower limb extremities[ncbi.nlm.nih.gov]
  • Symptoms of moderate to severe RHD can include chest pain, breathlessness with physical activity or when lying down, weakness and tiredness, and swelling of the legs and face.[rhdaustralia.org.au]
  • People with rheumatic heart disease may experience shortness of breath, chest pain, and swelling of the joints.[mainlinehealth.org]
  • Symptoms Your child may have signs and symptoms that affect the heart, including: Trouble breathing Chest pain Swelling (edema) of the feet and ankles Heart murmur Your child may also have other signs and symptoms of rheumatic fever.[stlouischildrens.org]
  • The most common symptoms of the condition include palpitations, chest pain, breathlessness, swelling of joints, fever, muscle pain, increased heart rate and rashes on torso.[symptoma.com]
Heart Murmur
  • The blood culture results were positive for Streptococcus pyogenes, and we started treatment using ampicillin and clindamycin, although subsequent auscultation revealed a new-onset heart murmur.[ncbi.nlm.nih.gov]
  • Most people with RHD have a heart murmur which can be heard through a stethoscope.[rhdaustralia.org.au]
  • In children with rheumatic heart disease, doctors can often hear a heart murmur. During the exam, your child’s doctor will look for signs of inflammation in your child’s joints.[seattlechildrens.org]
  • Among the most serious signs is the development of a heart murmur and cardiac decompensation. Heart Damage . The seriousness of rheumatic fever lies primarily in the permanent damage it can do to the heart.[medical-dictionary.thefreedictionary.com]
  • Heart murmur CHF 13. VI. Laboratory and Diagnostic Test There is no diagnostic studies are specific for rheumatic heart disease, but the following can support the diagnosis: 14. WBC count and ESR is elevated C- reactive protein is positive.[slideshare.net]
  • Pregnancy is associated with many hemodynamic changes including expanded intravascular volume, tachycardia, increased intracardiac dimensions, and valvular regurgitation.[ncbi.nlm.nih.gov]
  • She presented with supraventricular tachycardia and died in the coronary care unit 4 weeks postpartum. The second case was a 28-year-old who was on warfarin for a mechanical mitral valve.[ncbi.nlm.nih.gov]
  • Carditis – manifestations of carditis include systolic murmur and tachycardia. Some patients may develop congestive heart failure with dyspnea, ascites, hepatomegaly and chest pain as indications.[symptoma.com]
Diastolic Murmur
  • The murmur is described as a mid-diastolic murmur heard at the mitral listening post with the bell of the stethoscope with the patient in the left lateral decubitus position at end expiration.[healio.com]
  • A few years later in 1835 James Hope (1801–1841) an English physician known for discovering the early diastolic murmur of mitral stenosis in 1829, who has been called “the first cardiologist” according to Wikipedia [12] described murmurs that originated[heartviews.org]
  • Other areas of note included streptococcal infections and rheumatic diseases (which, in addition to rheumatic fever, also highlighted arthritis and juvenile arthritis).[ncbi.nlm.nih.gov]
  • The constellation of signs and symptoms required to make the diagnosis include arthritis, carditis, subcutaneous nodules, rash, and Sydenham chorea. - arthritis: the arthritis is typically symmetrical and involves large joints such as the knees, ankles[childrensheartinstitute.org]
  • Human infection with group C Streptococcus is extremely rare and a select number of cases have been reported to cause acute pharyngitis, acute glomerulonephritis, skin and soft tissue infections, septic arthritis, osteomyelitis, pneumonitis, and bacteremia[ncbi.nlm.nih.gov]
  • Postmortem examination confirmed rheumatoid arthritis and rheumatic heart disease in this case.[ci.nii.ac.jp]
  • Carditis occurs in approximately 50 percent of those who have rheumatic fever Arthritis: swelling, redness and pain in the joints, especially knees, ankles, elbows and wrists.[chop.edu]
  • "Minor" criteria include arthralgia (joint pain - not arthritis, which implies inflammation of the joint), fever, bloodwork suggestive of increased inflammation (elevated ESR or CRP - "inflammatory markers"), and abnormalities on ECG.[childrensheartinstitute.org]
  • Minor manifestations of the disease are fever, arthralgia, long PR interval, and elevated acute phase reactant, elevated levels of leucocytes and presence of C-reactive protein.[symptoma.com]
Migratory Polyarthritis
  • Migratory polyarthritis is the most common symptom in acute rheumatic fever. Subcutaneous nodules arise over the bones and tendons, as well as a rash that starts on the trunk and extends to the limbs.[healio.com]
  • KEYWORDS: Atrial fibrillation; Hemorrhagic transformation; Leukoaraiosis; Rheumatic heart disease; Stroke[ncbi.nlm.nih.gov]
  • Previous stroke and older age were independent predictors of stroke/transient ischemic attack or systemic embolism.[ncbi.nlm.nih.gov]
  • These clots then break off (embolise) and cause blockage in blood vessels in the brain and stroke.[betterhealth.vic.gov.au]
  • These complications imply long-standing disease with further potential for additional consequences such as stroke, commonly caused by rheumatic heart disease in the developing world.[blog.oup.com]
  • However, whether ALP levels play a role in HT after stroke remains an open question, especially in cardioembolic stroke patients.[ncbi.nlm.nih.gov]
  • Sydenham's chorea is the most common type of acquired chorea in childhood which is a major neurological manifestation of rheumatic fever.[ncbi.nlm.nih.gov]
  • Of nine patients with Sneddon's syndrome, three had rheumatic heart disease (mitral valve stenosis, regurgitation, or both) due to rheumatic fever or Sydenham's chorea.[ncbi.nlm.nih.gov]
  • Chorea – this is characterized by choreiform movements which increase during stress. This is more commonly seen in adults, particularly with rheumatic fever.[symptoma.com]
  • Group C Streptococcus is rarely associated with rheumatic heart disease and most children exhibiting acute onset of common symptoms, such as chorea, fever, carditis, and rash (erythema marginatum) will present to the emergency department first.[ncbi.nlm.nih.gov]
  • "Major" criteria include those listed above (arthritis, carditis, subcutaneous nodules, rash, Sydenham chorea).[childrensheartinstitute.org]


Streptococcal infection is confirmed by measuring anti-streptococcal antibody levels which tend to be elevated. This is important especially in those patients who have chorea as the only major diagnostic manifestation. Rapid antigen detection test is used in the diagnosis of streptococcal pharyngitis. This along with throat culture are confirmatory tests for streptococcal infection, which is an antecedent for rheumatic heart disease. Inflammation brings about an increase in the levels of C-reactive protein and ESR and this is used to check for recurrence and also to monitor inflammation.
Chest radiography helps to differentiate symptoms of heart failure from that of rheumatic disorders. It helps to diagnose manifestations like cardiomegaly and pulmonary congestion. Valve disorders and ventricular dysfunction are diagnosed with echocardiography. This imaging technique help in better diagnosis of the condition when compared to clinical findings alone [8]. It is also used in monitoring the progression of valve stenosis and may be useful in deciding its best treatment modality. Electrocardiography was found to be 92% specific in the diagnosis of rheumatic heart disease [9]. Mitral and aortic valve diseases are assessed by using heart catheterization. Some patients may show first degree atrioventricular block indicated by the prolongation of PR interval in ECG.
Histological tests help to reveal the presence of lesions on valves at the line of closure. Presence of Aschoff bodies or nodules of scar tissue can be noted in the pericardium. Microscopic examination of the affected valve show presence of thrombi and inflammation. Chronic form of rheumatic heart disease may have neovascularization and calcification in the different wall layers [10]. Immunohistochemistry tests may reveal the presence of elevated levels of CD4 and CD8 T-cells.

Pericardial Effusion
  • Echocardiography in a 23-year-old woman with chronic rheumatic heart disease revealed severe mitral and tricuspid stenosis, severe tricuspid regurgitation with severe pulmonary hypertension, and mild-to-moderate pericardial effusion that resulted in cardiac[ncbi.nlm.nih.gov]
  • Other complications were pulmonary hypertension and pericardial effusion. Conclusion: Rheumatic heart disease is still prevalent among children in Lagos although the prevalence is reducing.[ncbi.nlm.nih.gov]
  • effusion myocardial inflammation Promoted articles (advertising)[radiopaedia.org]
  • Echocardiography helps evaluate valvular damage, chamber size, ventricular function and the presence of a pericardial effusion. Cardiac catheter evaluates valvular damage and left ventricular function in severe cardiac dysfunction. 16.[slideshare.net]
Prolonged PR Interval
  • ECG reveals no diagnostic changes, but 20% of patient show a prolonged PR interval. 15. Echocardiography helps evaluate valvular damage, chamber size, ventricular function and the presence of a pericardial effusion.[slideshare.net]
Myocardial Fibrosis
  • While valvular involvement of rheumatic heart disease can be easily detected with conventional echocardiography by evaluating morphologic change of the valve, it still remains problematic to determine whether there is myocardial fibrosis after rheumatic[ncbi.nlm.nih.gov]
Aschoff Body
  • (WC/Nephron) Aschoff body (WC/Uthman) Anitschkow myocytes (WC/Uthman) IHC Features (Aschoff bodies & Anitschkow cells): [6] S100 -ve. Muscle specific actin -ve. Desmin -ve. NF -ve. Vimentin ve. CD45 ve (weak). See also Heart valves.[librepathology.org]
  • Presence of Aschoff bodies or nodules of scar tissue can be noted in the pericardium. Microscopic examination of the affected valve show presence of thrombi and inflammation.[symptoma.com]
  • It is characterized by the formation of granulomatous lesions called Aschoff bodies usually in the heart tissue.[fpnotebook.com]
  • Rheumatic heart disease rheumatic heart disease Cardiology A condition characterized in the acute form by myocarditis–which may cause atrial arrhythmias, 'bread and butter'-type serofibrinous pericarditis, Aschoff bodies in subserosal fibroadipose tissue–which[medical-dictionary.thefreedictionary.com]
  • Aschoff bodies are specific for postrheumatic carditis, whereas Anitschkow cells can be seen in a variety of conditions.[emedicine.medscape.com]


Preventing rheumatic fever due to group A streptococcal infection is often the first line of treatment. In patients who have already developed rheumatic heart disease, treatment focuses on treating streptococcal infection, suppressing inflammation of tissues, and treatment of congestive heart failure. This is followed by treatment to prevent recurrent rheumatic heart disease and associated complications. Streptococcal infection is treated with oral penicillin, ampicillin or amoxicillin. For those who are allergic to penicillin, erythromycin or first generation cephalosporin is recommended.
Inflammatory manifestations of the disease are controlled using salicylates and steroids. Aspirin is the most common medication suggested for improving inflammatory response, except in those patients with chorea. Once the symptoms improve, aspirin dose is gradually reduced. Phenobarbital and diazepam are useful in chorea, which often resolves on its own without any specific treatment. Oral prednisone is useful in patients with severe form of carditis, characterized by cardiomegaly and congestive heart failure. Depending on the severity of symptoms, oral prednisone may be continued for two to six weeks. Digoxin and diuretics are recommended for patients with acute rheumatic fever. In valve insufficiency, cardiac output can be improved using ACE inhibitors.
Further damage to the valves should be prevented in case of acute rheumatic heart disease. Benzathine penicillin G intramuscular injections are the first line of prophylaxis treatment for the patients. This dose is given every four weeks and is continued for 5 years in patients with rheumatic fever without carditis [11]. Those with rheumatic fever and carditis, but without any symptoms of valve disorder, are recommended antibiotic treatment for 10 years.
Surgery is recommended in patients who show persisting symptoms even after treatment for acute rheumatic heart disease. This will in help in improving the functioning of the valves. Mitral valvulotomy, balloon valvuloplasty or mitral valve replacement are suggested in these cases. Some patients may require neurology and/or cardiothoracic consultation depending on the complications.


Mortality rate for rheumatic heart disease ranges from 1.8 in the US to 7.6 in the developing regions of South East Asia. Progress of the disease differs in different individuals. Prognosis often depends on the involvement of heart. The favorable factors for good prognosis in patients with mitral valve disease include little or no mitral valve regurgitation and absence of aortic valve lesion. But patients who have had more than one attack of rheumatic fever, with aortic or tricuspid lesion and atrial fibrillation might not have good prognosis. Those with mitral valve prolapse do not have good outcome even with surgical repair. This is due to scarring in leaflets and chords. In many case scarring remains progressive even with surgery. Good cardiac muscle functioning and incompetence without stenosis are very favorable for good prognosis in patients with aortic valve disease. But stenosis combined with incompetence and left ventricular hypertrophy are poor prognostic factors in aortic valve disease. In most of the cases the disease progresses step by step.


Group A streptococcal infection is thought to trigger an autoimmune response after 2-3 weeks of onset of pharyngitis [2]. This autoimmune reaction, which includes both cellular- and humoral-mediated immunity, is considered to be the cause of rheumatic heart disease. Streptococcal invasion of the upper respiratory epithelial cells results in symptoms of pharyngitis including sore throat, fever, headache and an increase in serum leukocyte levels. After few weeks from the onset of the condition, the infectious organism incites an acute inflammatory response, characteristic of rheumatic heart disease. Only infection of pharynx by the bacteria is found to cause this condition. Certain populations are found to be more susceptible to develop rheumatic heart disease with streptococcal infections [3]. This shows the probable presence of a genetic factor that increases the risk of this disease. A human leukocyte antigen (HLA) subtype DR (HLA-DR) is also known to be associated with the development of rheumatic heart disease [4].


Incidence of this disease reduced considerably by 20th century in the developed countries and is now in the range of one in 100,000 persons [5]. But in developing countries, this disease still has a higher incidence. Streptococcal pharyngitis is one of the most common etiological factor for this condition. Incidence of streptococcal infection may vary between countries and even within the same country. About 282,000 new cases of rheumatic heart disease are reported in the world every year [6].
It is a major reason for concern among children and young adults in developing countries, as it is most commonly seen in the age group of 5-15 years. Streptococcal pharyngitis is not common among children below the age of three years. A higher incidence of the disease is reported among Native Hawaiian and Maori population. Rate of incidence of rheumatic heart disease is found to be equal among men and women, but outcome is found to be better for men when compared to women. The median age for the occurrence of this disease is around 10 years, although it may also occur in some adults above the age of 25 years.
Rheumatic heart disease occurs in 39% of the patients with rheumatic fever. It is also associated with poverty. Poverty is related to poor housing and overcrowding, two factors that favor the spread of streptococcal infection among the inmates. Poverty also lead to lack of proper healthcare and under-nutrition, both of which may lead to complications associated with streptococcal infection.

Sex distribution
Age distribution


The actual pathogenesis of the disease is not fully understood yet. It is known that streptococcal pharyngitis and rheumatic fever are associated with rheumatic heart disease. One of the theories suggest that antibodies against the bacteria trigger a type II cytotoxic hypersensitivity reaction [2]. The cardiac and smooth muscle cells of the body are mostly affected as they have elements which are structurally similar to bacteria. This leads to the immune-mediated inflammatory condition - rheumatic fever. All major valves of the heart, including the mitral and aortic valves, are affected by the autoimmune response that develops after the infection. In acute form of rheumatic disease small thrombi develop in the valves, while in chronic condition valves might undergo thickening and fibrosis.
As cardiac cells are the most affected, it leads to pancarditis characterized by inflammation of all three cardiac layers – endocardium, myocardium and pericardium. In about half of the patients, carditis may occur. Pericarditis usually ensues in about 10% of the patients. 


Diagnosis and treatment of group A streptococcal pharyngitis is the most important method for preventing rheumatic heart disease. Treating rheumatic fever with antibiotics should help to prevent further streptococcal infections and also valve damage.


Rheumatic heart disease refers to a group of heart disorders that develop as a response to group A streptococci infection. The actual pathogenesis of the disease is not fully defined yet. The autoimmune reaction triggered by the infection result in cardiac inflammation and scarring. Rheumatic heart disease may be acute or chronic, and both the conditions lead to many complications. It is more prevalent in children in the age group of 5-15 years, particularly in developing countries including sub-Saharan Africa, south-central Asia, and also among the indigenous populations in Australia and New Zealand. It is common in regions where pharyngitis is not treated completely, either due to lack of compliance on the patient’s side or due to non-availability of antibiotics [1]. Rheumatic heart disease can be prevented by treating streptococcal infection. Mortality rate associated with the disease is about 1-10%.

Patient Information

Rheumatic heart disease refers to a group of inflammatory heart disorders that develop from rheumatic fever. This condition is very common among children in the age group of 5-15 years, particularly in developing countries. Rheumatic fever is caused by the infection of the bacteria streptococci.
Rheumatic fever affects heart, joints and the central nervous system. When left untreated, it may lead to valve damage, and thus, heart failure and death. Poverty increases the risk of developing this condition as it leads to poor housing and overcrowding. Lack of access to healthcare and availability of antibiotics also are important risk factors for rheumatic heart disease.
Rheumatic heart disease may not have always obvious symptoms. The most common symptoms of the condition include palpitations, chest pain, breathlessness, swelling of joints, fever, muscle pain, increased heart rate and rashes on torso. Diagnosis of the disease is done using a complete medical examination, imaging techniques, and medical history. Presence of past streptococcal infection is indicative of rheumatic heart disease if appropriate symptoms are present. Enlargement of heart is checked using echocardiogram and chest X-ray. Echocardiograms also help in identifying the structure of valves. Changes in the normal rhythm of the heart can be checked using ECG.
Treatment of rheumatic heart disease depends on the severity of the symptoms. Antibiotics are the first line of treatment to control streptococcal infection. Inflammation is controlled using medications like aspirin. Steroids may be recommended for those who have congestive heart failure. Treatment may be continued for 5 – 10 years depending on the risk of recurrence. Surgery is suggested if there is no improvement in symptoms even after standard treatment. Diagnosis and treatment of strep throat is the most important method to prevent rheumatic heart disease. Once diagnosed, infection should be treated to prevent further complications.



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Last updated: 2019-07-11 20:45